International consensus criteria for behavioural variant FTD (FTDC)
| I. Neurodegenerative disease |
| The following symptom must be present to meet criteria for bvFTD |
| A. Shows progressive deterioration of behaviour and/or cognition by observation or history (as provided by a knowledgeable informant). |
| II. Possible bvFTD |
| Three of the following behavioural/cognitive symptoms (A–F) must be present to meet criteria. Ascertainment requires that symptoms be persistent or recurrent, rather than single or rare events. |
| A. Early* behavioural disinhibition [one of the following symptoms (A.1–A.3) must be present]: |
| A.1. Socially inappropriate behaviour |
| A.2. Loss of manners or decorum |
| A.3. Impulsive, rash or careless actions |
| B. Early apathy or inertia [one of the following symptoms (B.1–B.2) must be present]: |
| B.1. Apathy |
| B.2. Inertia |
| C. Early loss of sympathy or empathy [one of the following symptoms (C.1–C.2) must be present]: |
| C.1. Diminished response to other people’s needs and feelings |
| C.2. Diminished social interest, interrelatedness or personal warmth |
| D. Early perseverative, stereotyped or compulsive/ritualistic behaviour [one of the following symptoms (D.1–D.3) must be present]: |
| D.1. Simple repetitive movements |
| D.2. Complex, compulsive or ritualistic behaviours |
| D.3. Stereotypy of speech |
| E. Hyperorality and dietary changes [one of the following symptoms (E.1–E.3) must be present]: |
| E.1. Altered food preferences |
| E.2. Binge eating, increased consumption of alcohol or cigarettes |
| E.3. Oral exploration or consumption of inedible objects |
| F. Neuropsychological profile: executive/generation deficits with relative sparing of memory and visuospatial functions [all of the following symptoms (F.1–F.3) must be present]: |
| F.1. Deficits in executive tasks |
| F.2. Relative sparing of episodic memory |
| F.3. Relative sparing of visuospatial skills |
| III. Probable bvFTD |
| All of the following symptoms (A–C) must be present to meet criteria. |
| A. Meets criteria for possible bvFTD |
| B. Exhibits significant functional decline (by caregiver report or as evidenced by Clinical Dementia Rating Scale or Functional Activities Questionnaire scores) |
| C. Imaging results consistent with bvFTD [one of the following (C.1–C.2) must be present]: |
| C.1. Frontal and/or anterior temporal atrophy on MRI or CT |
| C.2. Frontal and/or anterior temporal hypoperfusion or hypometabolism on PET or SPECT |
| IV. Behavioural variant FTD with definite FTLD Pathology |
| Criterion A and either criterion B or C must be present to meet criteria. |
| A. Meets criteria for possible or probable bvFTD |
| B. Histopathological evidence of FTLD on biopsy or at post-mortem |
| C. Presence of a known pathogenic mutation |
| V. Exclusionary criteria for bvFTD |
| Criteria A and B must be answered negatively for any bvFTD diagnosis. Criterion C can be positive for possible bvFTD but must be negative for probable bvFTD. |
| A. Pattern of deficits is better accounted for by other non-degenerative nervous system or medical disorders |
| B. Behavioural disturbance is better accounted for by a psychiatric diagnosis |
| C. Biomarkers strongly indicative of Alzheimer’s disease or other neurodegenerative process |
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